Hematopathology Practice Questions

Q: What type of red blood cells (RBCs) are typically seen in chronic renal failure?

Normocytic. ### Explanation **Correct Option: C. Normocytic** The anemia associated with **Chronic Renal Failure (CRF)** is typically **Normocytic Normochromic Anemia**. **Why it is correct:** The primary pathophysiology in CRF is the **deficiency of Erythropoietin (EPO)** [1]. EPO is a glycoprotein hormone produced by the peritubular interstitial cells of the kidney in response to hypoxia [1], [2]. In chronic kidney disease, the functional renal mass decreases, leading to inadequate EPO production [1]. Since the bone marrow is healthy but lacks the hormonal signal to produce more cells, the RBCs produced are normal in size (normocytic) and color (normochromic), but their absolute number is decreased. Other contributing factors include the accumulation of uremic toxins (which shorten RBC lifespan) and chronic inflammation (Anemia of Chronic Disease). **Why other options are incorrect:** * **A. Microcytic:** This is characteristic of Iron Deficiency Anemia (IDA) or Thalassemia. While CRF patients can develop IDA due to hemodialysis blood loss or poor absorption, the *primary* anemia of renal failure itself is normocytic. * **B. Macrocytic:** This is seen in Vitamin B12 or Folic acid deficiency. While some drugs used in renal patients might interfere with folate metabolism, it is not the classic presentation of renal anemia. **High-Yield Clinical Pearls for NEET-PG:** * **Target Hemoglobin:** In patients on Erythropoiesis-Stimulating Agents (ESAs), the target Hb is usually **10–11.5 g/dL**. Aiming for normal levels (>13 g/dL) increases the risk of stroke and cardiovascular events (CHOIR and CREATE trials). * **Burr Cells (Echinocytes):** These are small, spiked RBCs often seen on the peripheral smear of patients with uremia/CRF. * **First step in management:** Before starting EPO therapy, always ensure **iron stores are adequate** (Transferrin saturation >20% and Ferritin >100 ng/mL).

Q: The MYD88 L265P mutation is characteristically seen in which of the following hematological malignancies?

Waldenstrom Macroglobulinemia. ### Explanation **Correct Answer: B. Waldenstrom Macroglobulinemia** **Mechanism:** The **MYD88 L265P** mutation is a gain-of-function mutation found in over **90-95%** of patients with Waldenstrom Macroglobulinemia (WM). MYD88 is an adapter protein in the Toll-like receptor (TLR) and Interleukin-1 receptor signaling pathways. The L265P mutation (substitution of proline for leucine at position 265) leads to constitutive activation of the **NF-̀B pathway**, promoting the survival and proliferation of malignant lymphoplasmacytic cells. **Analysis of Incorrect Options:** * **A. Hairy Cell Leukemia:** The hallmark genetic driver here is the **BRAF V600E** mutation (seen in >95% of cases). * **C. Multiple Myeloma:** Characterized by complex cytogenetics, most commonly involving **IGH translocations** [e.g., t(11;14), t(4;14)] or 13q deletions, rather than MYD88 mutations. * **D. Acute Myeloid Leukemia (AML):** Associated with mutations in **FLT3, NPM1, or DNMT3A**, and various chromosomal translocations [e.g., t(8;21), t(15;17)]. **High-Yield Clinical Pearls for NEET-PG:** * **Waldenstrom Macroglobulinemia (WM):** Defined as Lymphoplasmacytic Lymphoma (LPL) with bone marrow involvement and an **IgM monoclonal gammopathy** [1]. * **Clinical Triad:** Hyperviscosity syndrome (visual disturbances, neurological symptoms), hepatosplenomegaly, and lymphadenopathy [1]. * **Diagnostic Clue:** Absence of lytic bone lesions (unlike Multiple Myeloma) and presence of **Dutcher bodies** (intranuclear inclusions of immunoglobulins). * **Therapeutic Target:** The presence of MYD88 mutation makes these patients highly responsive to **Ibrutinib** (a BTK inhibitor). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 609-610.

Q: A 4-year-old African boy develops a rapidly enlarging mass that involves the right side of his face. Biopsies of this lesion are shown below. Which chromosomal translocation is associated with this neoplasm?

c-myc. ***c-myc*** - The clinical presentation of a rapidly enlarging facial mass in an **African child** is classic for **endemic Burkitt lymphoma**, which is associated with the **t(8;14) translocation** involving the **c-myc oncogene**. - Endemic Burkitt lymphoma characteristically presents as **jaw and facial bone tumors** in children from **malaria-endemic regions** of Africa, with the **c-myc translocation** being the hallmark genetic abnormality. *bcl-2* - The **bcl-2 oncogene** is associated with **follicular lymphoma** and some cases of **diffuse large B-cell lymphoma** through the **t(14;18) translocation**. - **bcl-2** prevents **apoptosis** and is typically seen in **indolent B-cell lymphomas** affecting adults, not rapidly growing pediatric facial masses. *N-myc* - **N-myc amplification** is characteristically associated with **neuroblastoma**, a common pediatric tumor arising from **neural crest cells**. - Neuroblastoma typically presents as **abdominal masses** or **adrenal tumors** in young children, not as facial bone lesions. *erb-B* - The **erb-B (HER2/neu) oncogene** is associated with **breast carcinoma** and **gastric adenocarcinoma** in adults. - **erb-B overexpression** is linked to **epithelial malignancies** and is not associated with **B-cell lymphomas** or pediatric hematologic malignancies.

Q: In hemolytic anemia, what changes occur in the skull bones?

Diploic space widening. In chronic hemolytic anemias (such as Thalassemia major and Sickle Cell Anemia), the fundamental process is **Compensatory Erythroid Hyperplasia** [1]. To compensate for the shortened lifespan of red blood cells, the bone marrow undergoes massive expansion to increase erythropoiesis [1]. ### Why the correct answer is right: * **Diploic Space Widening:** The skull is a major site of hematopoiesis in children. As the bone marrow expands, it pushes against the inner and outer tables of the skull, leading to a significant **widening of the diploic space** (the spongy bone layer between the tables). * **Radiological Correlation:** This expansion causes the characteristic **"Crew-cut" or "Hair-on-end" appearance** on X-ray, as new bone trabeculae are laid down perpendicular to the skull tables [1]. ### Why the incorrect options are wrong: * **Outer Table Thickening:** In reality, the expanding marrow causes **thinning of the outer table** due to pressure atrophy [1]. The inner table is usually spared. * **Diploic Space Narrowing:** This occurs in conditions of osteosclerosis or aging, not in hemolytic anemia where marrow hypercellularity demands more space. ### NEET-PG High-Yield Pearls: 1. **Thalassemia Major:** Shows the most classic "Hair-on-end" appearance due to ineffective erythropoiesis and extreme marrow expansion [1]. 2. **Chipmunk Facies:** Marrow expansion also occurs in the maxillary bones, leading to prominent cheekbones and malocclusion of teeth [1]. 3. **Extramedullary Hematopoiesis:** When the marrow cannot keep up, the liver and spleen take over, leading to hepatosplenomegaly [1]. 4. **Paranasal Sinuses:** These are often obliterated by the expanding marrow, except for the ethmoid sinuses. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 644-649.

Question 1

What type of red blood cells (RBCs) are typically seen in chronic renal failure?

Accepted Answer

Normocytic

Answer

Microcytic

Answer

Macrocytic

Answer

None of the above

Question 2

What is the most common carcinoma that causes splenic metastasis?

Accepted Answer

Carcinoma of the ovary

Answer

Carcinoma of the pancreas

Answer

Carcinoma of the stomach

Answer

Carcinoma of the cervix

Question 3

Which of the following causes of anemia is associated with a hypoplastic marrow?

Accepted Answer

Fanconi's Anemia

Answer

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Answer

Hypersplenism

Answer

Myelofibrosis

Question 4

Lacunar cells are characteristic of which of the following conditions?

Accepted Answer

Nodular sclerosing type of Hodgkin’s disease

Answer

Pleomorphic adenoma

Answer

Hurler’s syndrome

Answer

None of the above

Question 5

The MYD88 L265P mutation is characteristically seen in which of the following hematological malignancies?

Accepted Answer

Waldenstrom Macroglobulinemia

Answer

Hairy cell leukemia

Answer

Multiple Myeloma

Answer

Acute Myeloid Leukemia (AML)

Question 6

A 4-year-old African boy develops a rapidly enlarging mass that involves the right side of his face. Biopsies of this lesion are shown below. Which chromosomal translocation is associated with this neoplasm?

Accepted Answer

c-myc

Answer

bcl-2

Answer

N-myc

Answer

erb-B

Question 7

What is the cause of fragmented RBCs in peripheral blood?

Accepted Answer

Option 1, 2, and 4

Answer

Microangiopathic hemolytic anemia

Answer

Disseminated intravascular coagulation (DIC)

Answer

Malignant hypertension

Question 8

Which of the following is NOT true about Multiple Myeloma?

Accepted Answer

Protein casts in the urine are composed of complete immunoglobulin chains.

Answer

It involves clonal proliferation of plasma cells.

Answer

It is more common in individuals over 50 years of age.

Answer

Amyloidosis can be a complication.

Question 9

In hemolytic anemia, what changes occur in the skull bones?

Accepted Answer

Diploic space widening

Answer

Outer table thickening

Answer

Diploic space narrowing

Answer

None of the above

Question 10

CD19 positive, CD22 positive, CD103 positive monoclonal B-cells with bright kappa positivity were found to comprise 60% of the peripheral blood lymphoid cells on flow cytometric analysis in a 55-year-old man with massive splenomegaly and a total leucocyte count of 3.3 x 10^9/L. Which one of the following is the most likely diagnosis?

Accepted Answer

Hairy cell leukemia

Answer

Splenic lymphoma with villous lymphocytes

Answer

Mantle cell lymphoma

Answer

B-cell prolymphocytic leukemia

Hematopathology — MCQs

Hematopathology — MCQs

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